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BACKGROUND/PURPOSE: The purpose of this report was to describe the use of multimodal imaging to establish the diagnosis of Bartonella henselae -associated optic neuropathy in a patient who presented with a central scotoma without overt evidence of optic nerve involvement. METHODS: This was a case report. Main outcome measures included clinical, optical coherence tomography, and fluorescein angiography findings. OBSERVATIONS: A 72-year-old woman presented with a 3-day history of central scotoma in the left eye. Her examination was remarkable for faint exudation in the nasal macula of the left eye but was otherwise normal for her age. Spectral domain optical coherence tomography of the macula revealed mild thickening of the papillomacular bundle with scattered small cystoid spaces and several intraretinal exudates, none of which were visible clinically. Fluorescein angiography revealed localized leakage of the inferotemporal optic disc. When prompted, the patient recalled being scratched multiple times by her two pet kittens. Serial testing showed rising anti- B. henselae ( B. henselae ) immunoglobulin G antibody titers to 1:1,280, confirming the suspected diagnosis of B. henselae -associated optic neuropathy. CONCLUSION: Bartonella -associated optic nerve involvement can occur without overt evidence of optic disc swelling. Multimodal imaging can be used to suggest the diagnosis and support appropriate serologic testing.
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Bartonella henselae , Disco Óptico , Doenças do Nervo Óptico , Papiledema , Humanos , Feminino , Animais , Gatos , Idoso , Escotoma/diagnóstico , Escotoma/etiologia , Doenças do Nervo Óptico/complicações , Angiofluoresceinografia , Imagem Multimodal , Tomografia de Coerência ÓpticaRESUMO
PURPOSE: To report a case of Vogt-Koyanagi-Harada-like uveitis followed by melanoma-associated retinopathy with focal chorioretinal atrophy and subsequent choroidal neovascularization in a patient with metastatic cutaneous melanoma. METHOD: Case report. Main outcome measures include external photography, anterior segment photography, ophthalmoscopic examination, fundus photography, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, optical coherence tomography angiography, and electroretinography. RESULTS: A 68-year-old man with a history cutaneous melanoma presented with Vogt-Koyanagi-Harada-like uveitis. Work-up revealed a pelvic mass, which was excised and found to be metastatic melanoma. Two years later, the patient developed melanoma-associated retinopathy with focal chorioretinal atrophy and adjacent choroidal neovascularization. CONCLUSION: Patients with metastatic cutaneous melanoma can develop distinct and sequential paraneoplastic ocular complications. Onset of a Vogt-Koyanagi-Harada-like uveitis may be a good prognostic factor for survival in patients with metastatic cutaneous melanoma.
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Neovascularização de Coroide , Melanoma , Síndromes Paraneoplásicas Oculares , Neoplasias Cutâneas , Uveíte , Síndrome Uveomeningoencefálica , Masculino , Humanos , Idoso , Melanoma/complicações , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/patologia , Síndrome Uveomeningoencefálica/complicações , Síndrome Uveomeningoencefálica/diagnóstico , Síndromes Paraneoplásicas Oculares/diagnóstico , Angiofluoresceinografia , Atrofia , Melanoma Maligno CutâneoRESUMO
PURPOSE: To describe a case of nonsyndromic retinitis pigmentosa caused by presumed compound heterozygous A615T and T522M mutations in HGSNAT, characterized by bilateral cystoid macular edema and retinal neovascularization. METHODS: Case report. The patient underwent clinical evaluation, multimodal imaging, and next-generation panel sequencing. In silico analysis was performed with PolyPhen-2, SIFT, and MutationTaster. Segregation analysis was not available. RESULTS: A 35-year-old hypertensive man presented with nyctalopia, photopsia, and difficulty reading for six months. He had no family history of visual deficits. The best-corrected visual acuity was 20/25 in the right eye and 20/20 in the left eye. Examination revealed midperipheral bone spicules and macular neovascularization in both eyes. Multimodal imaging demonstrated cystoid macular edema, ellipsoid band loss outside the central macula, and leakage from the neovascularization in both eyes. Sequencing detected four mutations in three genes, including two heterozygous mutations in HGSNAT (c.1843G>A, p.A615T and c.1565C>T, p.T522M). A615T is a pathogenic, hypomorphic mutation. T522M has not been previously phenotypically described. It is predicted damaging by in silico analysis and occurs at a conserved position near the eighth transmembrane domain, adjacent to residues in which missense mutations result in protein misfolding. CONCLUSION: This is, to the best of our knowledge, the first reported case of retinal neovascularization in a case of nonsyndromic retinitis pigmentosa due to HGSNAT mutation. The T522M variant likely functions as a severe mutation alongside the hypomorphic A615T mutation. These findings expand the genotypic and phenotypic spectrum of nonsyndromic retinitis pigmentosa.
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Edema Macular , Doenças Retinianas , Neovascularização Retiniana , Retinose Pigmentar , Masculino , Humanos , Adulto , Edema Macular/etiologia , Edema Macular/genética , Retinose Pigmentar/diagnóstico , Retinose Pigmentar/genética , Mutação , Doenças Retinianas/complicações , Acetiltransferases/genéticaRESUMO
PURPOSE: To describe two cases of catastrophic, bilateral retinal vascular occlusion after intravitreal (IVT) bevacizumab injection. METHODS: Case series. Main outcome measures included clinical and fluorescein angiography findings. RESULTS: Case 1-A 65-year-old woman with calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasis syndrome developed acute, severe, bilateral visual loss 2 weeks after bilateral IVT bevacizumab injection for proliferative diabetic retinopathy. Examination and fluorescein angiography revealed moderate anterior chamber inflammation, bilateral perivascular retinal hemorrhages, and near total retinal vascular occlusion. Extensive testing revealed moderately elevated anti-B2 glycoprotein (antiphospholipid) antibodies. Case 2-An 85-year-old man with polymyalgia rheumatica and left eye exudative age-related macular degeneration experienced severe, bilateral, sequential visual loss in the left eye and then right eye approximately 3 weeks after IVT bevacizumab left eye injection. Examination revealed bilateral panuveitis, diffuse perivascular exudates, and intraretinal hemorrhages. Fluorescein angiography showed diffuse venous leakage. Extensive testing revealed an elevated antinuclear antibody and mildly elevated anticardiolipin antibody. CONCLUSION: Patients with underlying retinal vascular vulnerabilities may be at increased risk of catastrophic, bilateral retinal vascular occlusion after treatment with IVT bevacizumab. The moderate-to-severe intraocular inflammation in both cases and the contralateral involvement after unilateral IVT injection in Case 2 suggest a possible delayed immune-mediated mechanism.
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Inibidores da Angiogênese , Doenças Retinianas , Masculino , Feminino , Humanos , Recém-Nascido , Idoso , Idoso de 80 Anos ou mais , Bevacizumab , Inibidores da Angiogênese/uso terapêutico , Anticorpos Monoclonais Humanizados/efeitos adversos , Fator A de Crescimento do Endotélio Vascular , Doenças Retinianas/induzido quimicamente , Injeções Intravítreas , Angiofluoresceinografia , InflamaçãoRESUMO
PURPOSE: To describe a patient with metastatic clear cell renal cell carcinoma in remission on maintenance nivolumab therapy who developed late-onset ocular toxicity manifesting as creamy chorioretinal lesions with exudative retinal detachment concerning for choroidal metastasis. METHOD: Case report. Main outcome measures include ophthalmoscopic examination, fundus photography, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, spectral domain optical coherence tomography, and B-scan ultrasonography. RESULTS: A 49-year-old woman with a medical history of metastatic clear cell renal cell carcinoma in remission for two years after immunotherapy with four cycles of ipilimumab and nivolumab followed by maintenance nivolumab infusions developed lesions concerning for choroidal metastases in her right eye. Optical coherence tomography of the lesions revealed a bacillary layer detachment containing possible fibrinous exudate organized into layers and underlying choroidal thickening with chorioretinal folds. Later, choroidal thickening and chorioretinal folds also occurred in the left eye. Given that pan imaging detected no metastasis and the posterior segment abnormalities resolved after cessation of nivolumab and treatment with systemic corticosteroids, the patient was diagnosed with nivolumab-induced Vogt-Koyanagi-Harada-like uveitis. CONCLUSION: This case expands on the clinical spectrum of nivolumab-induced Vogt-Koyanagi-Harada-like uveitis, a condition that can also present with bacillary layer detachment mimicking an early choroidal metastasis, manifest asymmetrically in each eye, and develop after long-standing treatment.
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Bacillus , Carcinoma de Células Renais , Uveíte , Síndrome Uveomeningoencefálica , Feminino , Humanos , Pessoa de Meia-Idade , Síndrome Uveomeningoencefálica/tratamento farmacológico , Nivolumabe/efeitos adversos , Angiofluoresceinografia , Tomografia de Coerência ÓpticaRESUMO
Objective In this study, we aimed to investigate whether zinc provided in Age-Related Eye Disease Study 2 (AREDS2) vitamins is associated with a decreased risk of contracting coronavirus disease 2019 (COVID-19). Materials and methods We conducted a retrospective observational cohort study involving patients at a retina-only practice who were provided a questionnaire at each visit to assess whether they were symptomatic of or had contracted COVID-19. Those who answered yes to testing positive for COVID-19 were retrospectively analyzed and categorized based on their AREDS2 vitamin use, and a Pearson's chi-squared test was performed. Demographic data and past ocular history were also analyzed. Results A total of 8,426 unique patients, including 2,111 with a diagnosis of age-related macular degeneration (AMD), were seen from April 1, 2020, to April 9, 2021. A total of 110 patients (1.3%) reported contracting COVID-19 and had positive COVID-19 tests. The average age of those who had contracted COVID-19 in this study was 68.3 years; 51.8% were male, 30.1% had AMD, 28.2% had diabetic retinopathy, 24.5% had surgical retinal disease, 11.8% had retinal vascular disease, and 4.5% had other disease states. Of the COVID-19-positive patients, 27.3% (30/110) took AREDS2 vitamins, while 72.7% (80/110) patients did not. A chi-squared analysis was performed, which was not statistically significant (p=0.667). Conclusions Oral zinc supplementation, in the form of AREDS2 vitamins, is not associated with a protective effect against contracting COVID-19.
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BACKGROUND: Communication skills training (CST) remains poorly represented and prioritised in medical schools despite its importance. A systematic scoping review (SSR) of CST is proposed to better appreciate current variability in their structure, content, and assessment. This is to guide their future design in medical school curricula. METHODS: The Systematic Evidence-Based Approach (SEBA) was used to guide concurrent SSRs of teaching and assessment in CST. After independent database searches, concurrent thematic and content analysis of included articles were conducted separately. Resultant themes/categories were combined via the jigsaw perspective to provide a more holistic view of the data. These were then compared to tabulated summaries of the included articles to create funnelled domains. RESULTS: 52,300 papers were identified, 150 full-text articles included, and four funnelled domains were identified: Indications, Design, Assessment, and Barriers and Enablers of CST. CSTs confer numerous benefits to physicians and patients. It saw increased confidence, improved diagnostic capabilities and better clinical management, as well as greater patient satisfaction and treatment compliance. Skills may be divided into core, prerequisite competencies, and advanced skills pertinent to more challenging and nuanced scenarios - such as population or setting-specific situations. CST teaching and assessment modalities were found to align with Miller's Pyramid, with didactic teaching gradually infused with experiential approaches to enhance their understanding and integration. A plethora of CST frameworks, teaching and assessment methods were identified and are presented together. CONCLUSION: While variable in approach, content and assessment, CST in medical schools often employ stage-based curricula to instil competency-based topics of increasing complexity throughout medical school education. This process builds on the application of prior knowledge and skills, influencing practice and, potentially, the students' professional identity formation. In addition, the institution plays a critical role in overseeing training, ensuring longitudinal guidance and holistic assessments of the students' progress.
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Educação de Graduação em Medicina , Faculdades de Medicina , Competência Clínica , Comunicação , Currículo , HumanosRESUMO
BACKGROUND: Recognizing that physicians may struggle to achieve knowledge, skills, attitudes and or conduct at one or more stages during their training has highlighted the importance of the 'deliberate practice of improving performance through practising beyond one's comfort level under guidance'. However, variations in physician, program, contextual and healthcare and educational systems complicate efforts to create a consistent approach to remediation. Balancing the inevitable disparities in approaches and settings with the need for continuity and effective oversight of the remediation process, as well as the context and population specific nature of remediation, this review will scrutinise the remediation of physicians in training to better guide the design, structuring and oversight of new remediation programs. METHODS: Krishna's Systematic Evidence Based Approach is adopted to guide this Systematic Scoping Review (SSR in SEBA) to enhance the transparency and reproducibility of this review. A structured search for articles on remediation programs for licenced physicians who have completed their pre-registration postings and who are in training positions published between 1st January 1990 and 31st December 2021 in PubMed, Scopus, ERIC, Google Scholar, PsycINFO, ASSIA, HMIC, DARE and Web of Science databases was carried out. The included articles were concurrently thematically and content analysed using SEBA's Split Approach. Similarities in the identified themes and categories were combined in the Jigsaw Perspective and compared with the tabulated summaries of included articles in the Funnelling Process to create the domains that will guide discussions. RESULTS: The research team retrieved 5512 abstracts, reviewed 304 full-text articles and included 101 articles. The domains identified were characteristics, indications, frameworks, domains, enablers and barriers and unique features of remediation in licenced physicians in training programs. CONCLUSION: Building upon our findings and guided by Hauer et al. approach to remediation and Taylor and Hamdy's Multi-theories Model, we proffer a theoretically grounded 7-stage evidence-based remediation framework to enhance understanding of remediation in licenced physicians in training programs. We believe this framework can guide program design and reframe remediation's role as an integral part of training programs and a source of support and professional, academic, research, interprofessional and personal development.
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Medicina , Médicos , Atitude , Humanos , Reprodutibilidade dos TestesRESUMO
Purpose: To describe a patient with atypical Bartonella henselae (B. henselae)-associated ocular inflammation that manifested with recurrent, bilateral segmental periphlebitis. Observations: A 32-year-old White man presented with multiple paracentral scotomata in each eye. Examination revealed mild vitreous cell, segmental sheathing of the retinal veins, and inflammation of the paravenous retina in each eye. Multimodal imaging, including optical coherence tomography as well as widefield fundus autofluorescence, fluorescein angiography, and indocyanine green angiography, was consistent with bilateral, segmental retinal periphlebitis with paravenous inflammation and retinochoroidal scarring. Serology showed elevated B. henselae antibody titers, but was otherwise unrevealing, and the patient was diagnosed with presumed B. henselae-associated ocular inflammation. Treatment with systemic doxycycline (100 mg PO BID) for four weeks improved the patient's symptoms and posterior uveitis. However, after an asymptomatic period of nearly one year, his bilateral pericentral scotomata recurred and posterior segment examination confirmed new foci of retinal periphlebitis in each eye. Re-treatment with doxycycline (100 mg PO BID) for four weeks again yielded improvement, but one month after completing his antibiotic course, his visual symptoms recurred, and we observed additional areas of periphlebitis and paravenous retinitis with associated branch retinal vein occlusions in each eye. This time a dual antibiotic regimen of doxycycline (100 mg PO BID) and rifampin (300 mg PO BID) was administered for three months, with improvement. Over the next eight years, the patient experienced no further disease relapse, and the previous sites of retinal periphlebitis eventually developed perivenous fibrosis with paravenous retinochoroidal scarring. Conclusion: Rarely, patients with B. henselae-associated ocular inflammation develop segmental retinal periphlebitis with or without retinal vein occlusion. This form of ocular bartonellosis can recur, requiring multiple courses of antimicrobial therapy.
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PURPOSE: To investigate the clinical and anatomic characteristics of soccer ball-induced posterior segment injuries in the era of modern multi-modal imaging. METHODS: Retrospective case series of patients with soccer ball injury and diagnostic imaging from 2007 to 2020 at a single vitreoretinal practice. RESULTS: Eight patients met inclusion criteria. Fundus photographs (FP) and optical coherence tomography (OCT) were obtained in eight patients, fundus autofluorescence (FAF) in five patients, fluorescein angiography (FA) in three patients, and en-face OCT and OCT-Angiography (OCT-A) were obtained in two patients each. FA and FAF identified traumatic pigment epitheliopathy secondary to commotio. Increased hypo-autofluorescence was associated with shallow, peripheral retinal detachment on FAF. OCT of the macula displayed outer retinal changes associated with commotio, and offered insight into the acute and subacute changes of traumatic macular hole formation. A patient displayed foveal hyper-reflectivity in the shape of an hourglass with retinal cyst at the level of the external limiting membrane (ELM) as seen on OCT and En-face OCT. A patient with commotio involving the macula lacked microvascular changes on OCT-A. CONCLUSION: OCT, FA, and FAF imaging may aid in the work-up and management of the soccer ball-related posterior segment injuries.
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Traumatismos Oculares , Futebol , Traumatismos Oculares/diagnóstico por imagem , Traumatismos Oculares/etiologia , Angiofluoresceinografia/métodos , Humanos , Imagem Multimodal/métodos , Estudos Retrospectivos , Tomografia de Coerência Óptica/métodos , Acuidade VisualRESUMO
PURPOSE: To describe an atypical case of necrotizing herpetic retinitis (NHR) that presented initially with mild anterior uveitis, optic disc swelling, and peripapillary serous retinal detachment (SRD). OBSERVATIONS: A 48-year-old Asian Indian man presented with blurred central vision and pain in his left eye. Examination revealed mild anterior chamber inflammation, optic disc swelling, and a peripapillary SRD in the affected eye. Multimodal imaging, including widefield fluorescein angiography, showed optic disc leakage and confirmed the presence of a peripapillary SRD, but was otherwise unremarkable with no evidence of retinitis. The patient was diagnosed with presumed Vogt-Koyanagi-Harada disease and was treated with systemic corticosteroids. While there was objective visual improvement initially, the patient subsequently noted peripheral vision loss and was found to have peripheral necrotizing retinitis and occlusive retinal vasculitis in affected eye. Polymerase chain reaction-based testing of aqueous humor detected varicella zoster virus DNA, confirming the diagnosis of NHR. CONCLUSION AND IMPORTANCE: Optic disc edema and peripapillary SRD may precede the development of overt retinitis in some patients with NHR.
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BACKGROUND: The COVID-19 pandemic has changed the healthcare landscape drastically. Stricken by sharp surges in morbidity and mortality with resource and manpower shortages confounding their efforts, the medical community has witnessed high rates of burnout and post-traumatic stress amongst themselves. Whilst the prevailing literature has offered glimpses into their professional war, no review thus far has collated the deeply personal reflections of physicians and ascertained how their self-concept, self-esteem and perceived self-worth has altered during this crisis. Without adequate intervention, this may have profound effects on their mental and physical health, personal relationships and professional efficacy. METHODS: With mentions of the coronavirus pervading social media by the millions, this paper set out to collate and thematically analyse social media posts containing first-person physician reflections on how COVID-19 affected their lives and their coping mechanisms. A consistent search strategy was employed and a PRISMA flowchart was used to map out the inclusion/exclusion criteria. RESULTS: A total of 590 social media posts were screened, 511 evaluated, and 108 included for analysis. Salient themes identified include Disruptions to Personal Psycho-Emotional State, Disruptions to Professional Care Delivery, Concern for Family, Response from Institution, Response from Society and Coping Mechanisms. CONCLUSION: It is evident that the distress experienced by physicians during this time has been manifold, multi-faceted and dominantly negative. Self-concepts were distorted with weakened self-esteem and perceived self-worth observed. The Ring Theory of Personhood (RToP) was adopted to explain COVID-19's impact on physician personhood as it considers existential, individual, relational and social concepts of the self. These entwined self-concepts serve as 'compensatory' to one another, with coping mechanisms buffering and fortifying the physician's overall personhood. With healthcare institutions playing a vital role in providing timely and targeted support, it was further proposed that a comprehensive assessment tool based on the RToP could be developed to detect at-risk physicians and evaluate the presence and effectiveness of established support structures.
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COVID-19 , Médicos , Mídias Sociais , Humanos , Pandemias , SARS-CoV-2 , AutoimagemRESUMO
INTRODUCTION: Ensuring medical students are equipped with essential knowledge and portable skills to face complex ethical issues underlines the need for ethics education in medical school. Yet such training remains variable amidst evolving contextual, sociocultural, legal and financial considerations that inform training across different healthcare systems. This review aims to map how undergraduate medical schools teach and assess ethics. METHODS: Guided by the Systematic Evidence-Based Approach (SEBA), two concurrent systematic scoping reviews were carried out, one on ethics teaching and another on their assessment. Searches were conducted on PubMed, Embase, PsycINFO and ERIC between 1 January 1990 and 31 December 2020. Data was independently analysed using thematic and content analysis. RESULTS: Upon scrutinising the two sets of full-text articles, we identified 141 articles on ethics teaching and 102 articles on their assessments. 83 overlapped resulting in 160 distinct articles. Similar themes and categories were identified, these include teaching modalities, curriculum content, enablers and barriers to teaching, assessment methods, and their pros and cons. CONCLUSION: This review reveals the importance of adopting an interactive, multimodal and interdisciplinary team-teaching approach to ethics education, involving community resource partners and faculty trained in ethics, law, communication, professionalism, and other intertwining healthcare professions. Conscientious effort should also be put into vertically and horizontally integrating ethics into formal medical curricula to ensure contextualisation and application of ethics knowledge, skills and attitudes, as well as protected time and adequate resources. A stage-based multimodal assessment approach should be used to appropriately evaluate knowledge acquisition, application and reflection across various practice settings. To scaffold personalised development plans and remediation efforts, multisource evaluations may be stored in a centralised portfolio. Whilst standardisation of curricula content ensures cross-speciality ethical proficiency, deliberative curriculum inquiry performed by faculty members using a Delphi approach may help to facilitate the narrowing of relevant topics.
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Educação de Graduação em Medicina , Estudantes de Medicina , Currículo , Ética Médica , Humanos , Faculdades de MedicinaRESUMO
PURPOSE: To discuss the mechanism of injury and characterize the clinical features of ocular trauma associated with elastic cord exercise equipment band injuries in a consecutive series of patients seen at a single vitreoretinal surgery practice. METHODS: We performed a retrospective review of all patients who were treated for blunt trauma from 2013 to 2020 at a single vitreoretinal practice. RESULTS: Thirteen eyes from 11 patients met the inclusion criteria of possessing ocular trauma secondary to recoil from exercise bands. Presenting visual acuity ranged from 20/16 to HM (median: 20/32). The most frequently observed anterior segment pathologies were traumatic iritis (54%) and angle recession (31%). The most common posterior segment findings were vitreous hemorrhage (54%) and peripheral commotio retinae (54%). Three eyes (23%) required surgical intervention. Follow-up intervals ranged from 0 to 10 months (median: 1.75 months). Visual acuity at last examination ranged from 20/13 to 20/400 (median: 20/40). CONCLUSION: A wide spectrum of serious ocular injuries requiring medical and surgical intervention can result from this form of blunt ocular trauma. The frequency of this event would be decreased by the use of sports goggles and careful inspection of equipment for wear and over use.
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Traumatismos Oculares , Ferimentos não Penetrantes , Humanos , Traumatismos Oculares/diagnóstico , Traumatismos Oculares/complicações , Acuidade Visual , Hemorragia Vítrea/cirurgia , Ferimentos não Penetrantes/complicações , Ferimentos não Penetrantes/diagnóstico , Ferimentos não Penetrantes/terapia , Estudos RetrospectivosRESUMO
PURPOSE: To describe a case of amyloid A protein amyloidosis that produced an orbital inflammatory response with a novel presentation. METHODS: Case report. RESULTS: A 24-year-old Caucasian women with a history of intravenous heroin use was hospitalized for tricuspid valve endocarditis and methicillin-resistant Staphylococcus aureus bacteremia, as well as acute renal failure. She received hemodialysis and intravenous daptomycin and had negative blood cultures for 3 weeks, when she developed sudden bilateral orbital swelling and blurred vision. Visual acuity was 20/200 in the right eye and 20/400 in the left eye. Examination revealed proptosis, conjunctival chemosis and desiccation, optic disk swelling, creamy choroidal infiltrates, and inferiorly located exudative retinal detachments in both eyes. Multimodal imaging demonstrated thickening of the sclera, choroid, and choriocapillaris as well as outer retinal disruption, subretinal fluid, and deposits of hyperfluorescent debris within the choriocapillaris, outer retina, and vitreous. Oral prednisone at 60 mg per day resolved the choroidal infiltrates and exudative detachments. Persistent nephrotic syndrome called for a renal biopsy, which demonstrated amyloid A protein amyloidosis. CONCLUSION: Orbital and choroidal Amyloid A protein amyloidosis can induce a local inflammatory response manifesting as orbital swelling, papillitis, posterior scleritis, choroiditis, and exudative retinal detachment, which responds to steroid therapy. The underlying pathology is likely a reactive inflammatory, vasoocclusive process involving the choriocapillaris and orbital vasculature to the presence of amyloid fibrils.
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Amiloidose , Daptomicina , Endocardite , Oftalmopatia de Graves , Staphylococcus aureus Resistente à Meticilina , Descolamento Retiniano , Humanos , Feminino , Adulto Jovem , Adulto , Proteína Amiloide A Sérica , Prednisona , Amiloide , Heroína , Oftalmopatia de Graves/patologia , Corioide/patologia , Descolamento Retiniano/diagnóstico , Descolamento Retiniano/etiologia , Descolamento Retiniano/patologia , Endocardite/patologia , Amiloidose/complicações , Amiloidose/diagnóstico , Amiloidose/patologiaRESUMO
PURPOSE: To report a series of patients with occlusive retinal vasculitis associated with systemic sclerosis (SSc) and elevated antiphospholipid antibody titers. METHOD: Case series. Main outcome measures included clinical and fluorescein angiographic findings at presentation and over time. OBSERVATIONS: Case 1 - A 61-year-old woman initially diagnosed with idiopathic, bilateral panuveitis and retinal vasculitis causing peripheral nonperfusion was subsequently diagnosed with limited cutaneous systemic sclerosis (lcSSc). Her ocular inflammation and retinal vasculitis were controlled with topical and periocular corticosteroids, but she eventually developed peripheral retinal vascular occlusion that progressed to macular ischemia 11 years after presentation. Repeat serologic evaluation detected interval development of antiphospholipid antibodies. Case 2 - A 58-year-old woman was found to have bilateral peripheral nonperfusion and retinal neovascularization in her right eye. Given her elevated hemoglobin A1c of 8.5%, she was diagnosed with presumed proliferative diabetic retinopathy. Three years after initial presentation, she was diagnosed with lcSSc. Subsequent serum workup detected elevated B2-glycoprotein antibody titers. Her peripheral nonperfusion progressed despite adequate glycemic control, resulting in further neovascularization in each eye. Case 3 - A 40-year-old woman with diffuse cutaneous systemic sclerosis (dcSSc) and elevated titers of anti-cardiolipin antibodies developed multiple branch retinal artery occlusions with subsequent neovascularization of the retina, optic disc, and angle in the right eye. CONCLUSION AND IMPORTANCE: Vision-threatening occlusive retinal vasculitis may develop in select patients with SSc. The presence of elevated anti-phospholipid antibody titers may confer increased risk for this vision-threatening complication.
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PURPOSE: To provide a comprehensive review of rituximab use for the treatment of non-infectious/non-malignant orbital inflammation. METHODS: Review of literature through January 2021. RESULTS: Individual data was available for 167 patients with refractory non-infectious/non-malignant orbital inflammation who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (108/149, 72.5%) at a mean of 44.6 months following the diagnosis of orbital inflammation (range = 0 to 360 months; median = 13.7 months). Patients with non-infectious/non-malignant orbital inflammation either received prior treatment with corticosteroids only (27/122, 22.1%), or with one (31/122, 25.4%), two (25/122, 20.5%), or three or more (25/122, 20.5%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (80/144, 55.6%), followed by the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; 51/144, 35.4%). Various other off-label regimens were used infrequently (13/144, 9.0%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with orbital inflammation (146/166, 88.0%). Commonly treated diagnoses included granulomatosis with polyangiitis (99/167, 59.3%), IgG-4 related disease (36/167, 21.6%), and orbital inflammation of indeterminate cause (25/167, 15.0%). No side effects were reported in 83.3% (55/66) of cases. The most common RTX-induced adverse event was an infusion-related temporary exacerbation of orbital disease (4/66, 6.1%), which occurred prior to the routine use of systemic corticosteroids as pre-conditioning. CONCLUSIONS: Overall, RTX appears to be both efficacious and well-tolerated as second- or third-line therapy for patients with non-infectious/non-malignant orbital inflammation.
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PURPOSE: To provide a comprehensive review of rituximab use for the treatment of non-infectious uveitis and scleritis. METHODS: Review of literature through December 2020. RESULTS: Individual data was available for 229 patients with refractory non-infectious uveitis (n = 108) or scleritis (n = 121) who received treatment with rituximab (RTX). Rituximab was generally utilized as third-line or later treatment (uveitis: 67/90, 74.4%; scleritis: 90/96, 93.8%) at a mean of 33.5 months following the diagnosis of uveitis (range = 0 to 168.0 months; median = 24.0 months) and 39.4 months after diagnosis of scleritis (range = 1.0 to 168.0 months; median = 21.0 months). Patients with non-infectious uveitis and scleritis either received prior treatment with corticosteroids only (uveitis: 18/90, 20%; scleritis: 4/94, 4.3%), or with one (uveitis: 19/90, 21.1%; scleritis: 30/94, 31.9%), two (uveitis: 11/90, 12.2%; scleritis 27/94, 28.7%), or three or more (uveitis: 37/90, 41.1%; scleritis: 31/94, 33.0%) corticosteroid-sparing immunosuppressive agents with or without corticosteroids before initiation of RTX treatment. The rheumatologic protocol (two infusions of 1 gram of RTX separated by 14 days) was utilized most frequently (uveitis: 45/87, 51.7%; scleritis: 87/114, 76.3%), followed by the Foster protocol (eight weekly infusions of 375 mg/m2 RTX; uveitis: 18/87, 20.7%; scleritis: 10/114, 8.8%), and the oncologic protocol (four weekly infusions of 375 mg/m2 RTX; uveitis: 5/87, 5.7%; scleritis: 6/114, 5.3%). Various other off-label regimens were used infrequently (uveitis: 19/87, 21.8%; scleritis 11/114, 9.6%). Rituximab treatments resulted in a positive therapeutic response for the majority of patients with non-infectious uveitis (81/97, 83.5%). Commonly treated uveitic diagnoses included non-paraneoplastic autoimmune retinopathy (30/107, 28.0%), juvenile idiopathic arthritis (21/107, 19.6%), Vogt-Koyanagi-Harada disease (12/107, 11.2%), and Behçet disease (11/107, 10.3%). Cases of non-infectious scleritis were most commonly attributed to granulomatosis with polyangiitis (75/121, 62.0%) and rheumatoid arthritis (15/121, 12.4%), and showed an even greater rate of positive therapeutic response (112/120, 93.3%) following RTX treatment. No side effects were reported in 76.3% (74/97) of uveitis and 85.5% (71/83) scleritis cases. Of those cases associated with RTX-induced adverse events, the most common were infusion reactions of various severity (11/35, 31.4%). CONCLUSIONS: Overall, RTX appeared to be both effective and well-tolerated as second or third-line therapy for patients with non-infectious uveitis and scleritis.
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BACKGROUND: Molding competent clinicians capable of applying ethics principles in their practice is a challenging task, compounded by wide variations in the teaching and assessment of ethics in the postgraduate setting. Despite these differences, ethics training programs should recognise that the transition from medical students to healthcare professionals entails a longitudinal process where ethics knowledge, skills and identity continue to build and deepen over time with clinical exposure. A systematic scoping review is proposed to analyse current postgraduate medical ethics training and assessment programs in peer-reviewed literature to guide the development of a local physician training curriculum. METHODS: With a constructivist perspective and relativist lens, this systematic scoping review on postgraduate medical ethics training and assessment will adopt the Systematic Evidence Based Approach (SEBA) to create a transparent and reproducible review. RESULTS: The first search involving the teaching of ethics yielded 7669 abstracts with 573 full text articles evaluated and 66 articles included. The second search involving the assessment of ethics identified 9919 abstracts with 333 full text articles reviewed and 29 articles included. The themes identified from the two searches were the goals and objectives, content, pedagogy, enabling and limiting factors of teaching ethics and assessment modalities used. Despite inherent disparities in ethics training programs, they provide a platform for learners to apply knowledge, translating it to skill and eventually becoming part of the identity of the learner. Illustrating the longitudinal nature of ethics training, the spiral curriculum seamlessly integrates and fortifies prevailing ethical knowledge acquired in medical school with the layering of new specialty, clinical and research specific content in professional practice. Various assessment methods are employed with special mention of portfolios as a longitudinal assessment modality that showcase the impact of ethics training on the development of professional identity formation (PIF). CONCLUSIONS: Our systematic scoping review has elicited key learning points in the teaching and assessment of ethics in the postgraduate setting. However, more research needs to be done on establishing Entrustable Professional Activities (EPA)s in ethics, with further exploration of the use of portfolios and key factors influencing its design, implementation and assessment of PIF and micro-credentialling in ethics practice.